Manifestations and treatment of congenital fibrinogen deficiency

Ruchika Sharma; Bryce A. Kerlin

doi:10.1007/978-3-030-31661-7_5

Manifestations and treatment of congenital fibrinogen deficiency

Research output: Chapter in Book/Report/Conference proceeding › Chapter

Abstract

Fibrinogen is a complex glycoprotein that polymerizes following cleavage by thrombin to form the fibrin clot. Congenital fibrinogen disorders are heterogeneous and comprise qualitative and quantitative defects in fibrinogen. These may present as bleeding or thrombosis phenotypes. Congenital fibrinogen disorders result from mutations in any of the three genes (FGA, FGB, and FGG), and both autosomal dominant and recessive forms exist. Replacement therapy with fibrinogen concentrate may be warranted in some patients.

Original language	English (US)
Title of host publication	Pediatric Bleeding Disorders
Subtitle of host publication	A Clinical Casebook
Publisher	Springer International Publishing
Pages	51-58
Number of pages	8
ISBN (Electronic)	9783030316617
ISBN (Print)	9783030316600
DOIs	https://doi.org/10.1007/978-3-030-31661-7_5
State	Published - Jan 1 2020
Externally published	Yes

Keywords

Afibrinogenemia
Bleeding or thrombosis
Dysfibrinogenemia
Fibrinogen
Fibrinogen concentrate
Hypofibrinogenemia

ASJC Scopus subject areas

Medicine(all)

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10.1007/978-3-030-31661-7_5

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title = "Manifestations and treatment of congenital fibrinogen deficiency",

abstract = "Fibrinogen is a complex glycoprotein that polymerizes following cleavage by thrombin to form the fibrin clot. Congenital fibrinogen disorders are heterogeneous and comprise qualitative and quantitative defects in fibrinogen. These may present as bleeding or thrombosis phenotypes. Congenital fibrinogen disorders result from mutations in any of the three genes (FGA, FGB, and FGG), and both autosomal dominant and recessive forms exist. Replacement therapy with fibrinogen concentrate may be warranted in some patients.",

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AU - Kerlin, Bryce A.

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N2 - Fibrinogen is a complex glycoprotein that polymerizes following cleavage by thrombin to form the fibrin clot. Congenital fibrinogen disorders are heterogeneous and comprise qualitative and quantitative defects in fibrinogen. These may present as bleeding or thrombosis phenotypes. Congenital fibrinogen disorders result from mutations in any of the three genes (FGA, FGB, and FGG), and both autosomal dominant and recessive forms exist. Replacement therapy with fibrinogen concentrate may be warranted in some patients.

AB - Fibrinogen is a complex glycoprotein that polymerizes following cleavage by thrombin to form the fibrin clot. Congenital fibrinogen disorders are heterogeneous and comprise qualitative and quantitative defects in fibrinogen. These may present as bleeding or thrombosis phenotypes. Congenital fibrinogen disorders result from mutations in any of the three genes (FGA, FGB, and FGG), and both autosomal dominant and recessive forms exist. Replacement therapy with fibrinogen concentrate may be warranted in some patients.

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KW - Bleeding or thrombosis

KW - Dysfibrinogenemia

KW - Fibrinogen

KW - Fibrinogen concentrate

KW - Hypofibrinogenemia

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BT - Pediatric Bleeding Disorders

PB - Springer International Publishing

ER -

Manifestations and treatment of congenital fibrinogen deficiency

Abstract

Keywords

ASJC Scopus subject areas

Access to Document

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